중심방소엽을 침범하지 않은 반경련-반마비-간질증후군(Hemiconvulsion-hemiplegia-epilepsysyndrome)을 보인 환아 1례 : 초기 뇌 자기 공명 영상소견과 신경방사선학적 추적 관찰
Hemiconvulsion-hemiplegia-epilepsy Syndrome Sparing Paracentral Lobule : Early Magnetic Resonance Imaging Findings and Neuroradiological Follow-up
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HHE syndrome is characterized by hemiconvulsive seizure, hemiplegia and epilepsy occurring in sequence. Recently, cytotoxic edematous swelling of one hemisphere was presented as the pathogenesis because high signal intensity was shown in the diffusion image but no remarkable findings were noticed in the FLAIR image and T2WI in two cases presented by Freeman etc. The clinical course and the radiologic findings in our patient was the same as the above description other than sparing paracentral lobule. This reinforces the assumption that the brain damage in HHE syndrome is not induced by vascular hypoxic damage but by selectively cytotoxic damage through immunologic pathogenesis. Therefore, diffusion image is needed for the diagnosis of HHE syndrome in a febrile convulsive child. Also, early adequate treatment is required such as immunosuppressive treatment with high dose steroid and intravenous immunoglobulin. Furthermore, a proper measure to reduce cerebral edema is essential for the prevention of neurologic sequalae and deterioration to epilepsy.
Abstract
HHE syndrome is characterized by hemiconvulsive seizure, hemiplegia and epilepsy occurring in sequence. Recently, cytotoxic edematous swelling of one hemisphere was presented as the pathogenesis because high signal intensity was shown in the diffusion image but no remarkable findings were noticed in the FLAIR image and T2WI in two cases presented by Freeman etc. The clinical course and the radiologic findings in our patient was the same as the above description other than sparing paracentral lobule. This reinforces the assumption that the brain damage in HHE syndrome is not induced by vascular hypoxic damage but by selectively cytotoxic damage through immunologic pathogenesis. Therefore, diffusion image is needed for the diagnosis of HHE syndrome in a febrile convulsive child. Also, early adequate treatment is required such as immunosuppressive treatment with high dose steroid and intravenous immunoglobulin. Furthermore, a proper measure to reduce cerebral edema is essential for the prevention of neurologic sequalae and deterioration to epilepsy.
참고문헌 (15)
- Chauvel P., "Epileptic syndromes in infancy and adolescence", pp.277~293 (2005), Montrouge: John Libbey Eurotext Ltd
- Auvin S., "Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications", Seizure, 16, pp.371~376 (2007)
- Salih M.A., "Hemiconvulsion-hemiplegiaepilepsy syndrome. A clinical, electroencephalographic and neuroradiological study", Childs Nerv Syst, 13, pp.257~263 (1997)
- Yang S., "Two cases of hemiconvulsion- hemiplegia-epilepsy syndrome", J Korean Child Neurol Soc, 4, pp.238~243 (1997)
- Toldo I., "Hemiconvulsionhemipl-egiaepilepsy syndrome: early magnetic resonance imaging findings and neuroradiological followup", Brain Dev, 29, pp.109~111 (2007)
- "Acta Neurol Belg", 1962, 177~192
- "Rev Electroencephalogr Neurophysiol Clin", 1972, 5~28
- "Klin Padiatr", 2002, 126~127
- Sztriha L., "Regional cerebral blood perfusion in children with hemiplegia: A SPECT study", Neuropediatrics, 27, pp.178~183 (1996)
- Lee C., "Hemiconvulsion-hemiplegia-epilepsy syndrome as a presenting feature of L-2-hydroxyglutaric aciduria", J Child Neurol, 21, pp.538~540 (2006)
- Scantlebury M.H., "Association between factor V Leiden mutation and the hemiconvulsion, hemiplegia, and epilepsy syndrome: report of two cases", J Child Neurol, 17, pp.713~717 (2002)
- Mondal R.K., "Hemiconvulsion hemiplegia, epilepsy syndrome and inherited protein S deficiency", Indian J Pediatr, 73, pp.157~159 (2006)
- Freeman J.L., , J Child Neurol, 17, pp.10~16 (2002)
- Kimura M., "Hemiconvulsion- hemiplegia syndrome and elevated interleukin-6: case report", J Child Neurol, 17, pp.705~707 (2002)
- Mori Y., "Anatomopathology and pathogeny of the hemiconvulsion-hemiplegia-epilepsy syndrome", Part II. J Neurosurg Sci, 23, pp.1~22 (1979)